
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Glucagonoma natural history, complications and prognosis
Introduction
Overview
Overview
If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia. The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma. The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis. Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis. Age, grade, and distant metastases are the most significant predictors of survival.
Natural History
Natural History
- If left untreated, patients with glucagonoma may progress to develop necrolytic migratory erythema, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia.
- Glucagonoma has a very slow growth rate compared to most malignant tumors.
Complications
Complications
Complications of glucagonoma include:
- Metastasis:
- Glucagonomas are generally slow-growing but are usually advanced by the time of diagnosis.
- Metastasis occurs mainly in the liver but in few cases, it can occur in lymph nodes, peritoneum, lung, and adrenals.
- Weight loss: due to the catabolic effect of glucagon, most patients lose weight.
- Anemia
- Neuropsychiatric manifestations include depression, dementia, psychosis, and agitation
- Reversible dilated cardiomyopathy[1]
Prognosis
Prognosis
Prognosis of glucagonoma depends on the following:
- Whether or not the tumor can be removed by surgery.
- The stage of the tumor, the size of the tumor, whether cancer has spread outside the pancreas.
- The patient’s general health.
- Whether the tumor has just been diagnosed or has recurred.
- The presence of metastasis is associated with a particularly poor prognosis among patients with glucagonoma.
- The 10-year event free survival rate is less than 51.6% with metastasis and 64.3% without metastasis.
Additionally:
- Age, grade, and distant metastases are the most significant predictors of survival.
- Five and 10-year survival rates for patients undergoing resection of gastroenteropancreatic neuroendocrine tumors.[2]
- Sixty percent of glucagonomas are malignant. Once the tumor is metastatic, the cure is rare.[3]
References
References
- ↑ K. Chang-Chretien, J. T. Chew & D. P. Judge. “Reversible dilated cardiomyopathy associated with glucagonoma”. Heart (British Cardiac Society). 90 (7): e44. doi:10.1136/hrt.2004.036905. PMID 15201270.
- ↑ Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV (1996). “The glucagonoma syndrome. Clinical and pathologic features in 21 patients”. Medicine (Baltimore). 75 (2): 53–63. PMID 8606627.
- ↑ M. A. Chastain (2001). “The glucagonoma syndrome: a review of its features and discussion of new perspectives”. The American journal of the medical sciences. 321 (5): 306–320. PMID 11370794. Unknown parameter
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2], Mohammed Abdelwahed M.D[3]
